An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

Vitor Cavalcanti Trindade, Magda Carneiro-Sampaio, Eloisa Bonfa, Clovis Artur Silva, Vitor Cavalcanti Trindade, Magda Carneiro-Sampaio, Eloisa Bonfa, Clovis Artur Silva

Abstract

Childhood-onset systemic lupus erythematosus (cSLE) is a prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition. This disease is characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality. Racial/ethnic background, socioeconomic status, cost of medications, difficulty accessing health care, and poor adherence seem to impact lupus outcomes and treatment response. In this article, the management of cSLE patients is updated. Regarding pathogenesis, a number of potential targets for drugs have been studied. However, most treatments in pediatric patients are off-label drugs with recommendations based on inadequately powered studies, therapeutic consensus guidelines, or case series. Management practices for cSLE patients include evaluations of disease activity and cumulative damage scores, routine non-live vaccinations, physical activity, and addressing mental health issues. Antimalarials and glucocorticoids are still the most common drugs used to treat cSLE, and hydroxychloroquine is recommended for nearly all cSLE patients. Disease-modifying antirheumatic drugs (DMARDs) should be standardized for each patient, based on disease flare and cSLE severity. Mycophenolate mofetil or intravenous cyclophosphamide is suggested as induction therapy for lupus nephritis classes III and IV. Calcineurin inhibitors (cyclosporine, tacrolimus, voclosporin) appear to be another good option for cSLE patients with lupus nephritis. Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.

Conflict of interest statement

The authors declare no conflict of interest regarding this manuscript.

© 2021. The Author(s), under exclusive licence to Springer Nature Switzerland AG.

References

    1. Silva CA. Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know. Expert Rev Clin Immunol. 2016;12:907–910. doi: 10.1080/1744666X.2016.1195685.
    1. Silva CA, Aikawa NE, Pereira RMR, Campos LMA. Management considerations for childhood-onset systemic lupus erythematosus patients and implications on therapy. Expert Rev Clin Immunol. 2016;12:301–313. doi: 10.1586/1744666X.2016.1123621.
    1. Blay G, Rodrigues JC, Ferreira JCO, Leal GN, Gormezano NW, Novak GV, et al. Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome. Adv Rheumatol. 2018;58:39. doi: 10.1186/s42358-018-0038-4.
    1. Kahwage PP, Ferriani MPL, Furtado JM, de Carvalho LM, Pileggi GS, Gomes FHR, et al. Uveitis in childhood-onset systemic lupus erythematosus patients: a multicenter survey. Clin Rheumatol. 2017;36:547–553. doi: 10.1007/s10067-016-3534-0.
    1. Silva MF, Ferriani MP, Terreri MT, Pereira RM, Magalhães CS, Bonfá E, et al. A multicenter study of invasive fungal infections in patients with childhood-onset systemic lupus erythematosus. J Rheumatol. 2015;42:2296–2303. doi: 10.3899/jrheum.150142.
    1. Ferreira JCOA, Marques HH, Ferriani MPL, Gormezano NWS, Terreri MT, Pereira RM, et al. Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study. Lupus. 2015;25:754–759. doi: 10.1177/0961203315627203.
    1. Lube GE, Ferriani MPL, Campos LMA, Terreri MT, Bonfá E, Magalhães CS, et al. Evans syndrome at childhood-onset systemic lupus erythematosus diagnosis: a large multicenter study. Pediatr Blood Cancer. 2016;63:1238–1243. doi: 10.1002/pbc.25976.
    1. Marques VLS, Gormezano NWS, Bonfá E, Aikawa NE, Terreri MT, Pereira RM, et al. Pancreatitis subtypes survey in 852 childhood-onset systemic lupus erythematosus patients. J Pediatr Gastroenterol Nutr. 2016;62:328–334. doi: 10.1097/MPG.0000000000000990.
    1. Sakamoto AP, Silva CA, Ferriani MPL, Pereira RMR, Bonfá E, Saad-Magalhães C, et al. Characterization of chronic arthritis in a multicenter study of 852 childhood-onset systemic lupus erythematosus patients. Rheumatol Int. 2016;36:1641–1648. doi: 10.1007/s00296-016-3564-6.
    1. Sakamoto AP, Silva CA, Saad-Magalhães C, Alencar AN, Pereira RMR, Kozu K, et al. Stevens–Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study. Acta Reumatol Port. 2017;2017:250–255.
    1. Novak GV, Marques M, Balbi V, Gormezano NWS, Kozu K, Sakamoto AP, et al. Anti-RO/SSA and anti-La/SSB antibodies: Association with mild lupus manifestations in 645 childhood-onset systemic lupus erythematosus. Autoimmun Rev. 2017;16:132–135. doi: 10.1016/j.autrev.2016.12.004.
    1. Setoue DN, Pitta AC, Fiorot FJ, Nastri MM, Novak GV, Molinari BC, et al. Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: a Brazilian multicenter study. Autoimmun Rev. 2018;17:836–839. doi: 10.1016/j.autrev.2018.03.009.
    1. Thorbinson C, Oni L, Smith E, Midgley A, Beresford MW. Pharmacological management of childhood-onset systemic lupus erythematosus. Pediatr Drugs. 2016;18:181–195. doi: 10.1007/s40272-016-0170-8.
    1. Fiorot FJ, Islabão AG, Pereira RM, Terreri MT, Saad-Magalhães C, Novak GV, et al. Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity. Clin Rheumatol. 2019;38:2857–2863. doi: 10.1007/s10067-019-04631-0.
    1. Miguel DF, Terreri MT, Pereira RMR, Bonfá E, Silva CAA, Corrente JE, et al. Comparison of urinary parameters, biomarkers, and outcome of childhood systemic lupus erythematosus early onset-lupus nephritis. Adv Rheumatol. 2020 doi: 10.1186/s42358-020-0114-4.
    1. Islabão AG, Mota LMH, Ribeiro MCM, Arabi TM, Cividatti GN, Queiroz LB, et al. Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients. Autoimmun Rev. 2020 doi: 10.1016/j.autrev.2020.102693.
    1. Sakamoto AP, Silva CA, da Silva MFC, Lopes AS, Russo GCS, Sallum AME, et al. Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus. Rev Bras Reumatol. 2017;57:583–589. doi: 10.1016/j.rbre.2017.09.002.
    1. Pons-Estel BA, Bonfa E, Soriano ER, Cardiel MH, Izcovich A, Popoff F, et al. First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, Grupo Latino Americano de Estudio del Lupus)-Pan-American League of Associations of Rheumatology (PAN. Ann Rheum Dis. 2018;77:1549–1557. doi: 10.1136/annrheumdis-2018-213512.
    1. Ferreira JCOA, Trindade VC, Espada G, Morel Z, Bonfá E, Magalhães CS, et al. Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America. Clin Rheumatol. 2018;37:3299–3307. doi: 10.1007/s10067-018-4254-4.
    1. Gomes RC, Silva MF, Kozu K, Bonfá E, Pereira RM, Terreri MT, et al. Features of 847 childhood-onset systemic lupus erythematosus patients in three age groups at diagnosis: a Brazilian multicenter study. Arthritis Care Res. 2016;68:1736–1741. doi: 10.1002/acr.22881.
    1. Novak GV, Molinari BC, Ferreira JC, Sakamoto AP, Terreri MT, Pereira RMR, et al. Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study. Lupus. 2018;27:1712–1717. doi: 10.1177/0961203318787037.
    1. Lopes SRM, Gormezano NWS, Gomes RC, Aikawa NE, Pereira RMR, Terreri MT, et al. Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups. Lupus. 2017;26:996–1001. doi: 10.1177/0961203317690616.
    1. Ferriani MPL, Silva MFC, Pereira RMR, Terreri MT, Saad Magalhães C, Bonfá E, et al. Chronic spontaneous urticaria: a survey of 852 cases of childhood-onset systemic lupus erythematosus. Int Arch Allergy Immunol. 2015;167:186–192. doi: 10.1159/000438723.
    1. Anuardo P, Verdier M, Gormezano NWS, Ferreira GRV, Leal GN, Lianza A, et al. Subclinical pulmonary hypertension in childhood systemic lupus erythematosus associated with minor disease manifestations. Pediatr Cardiol. 2017;38:234–239. doi: 10.1007/s00246-016-1504-6.
    1. Esteves GCX, Gormezano NWS, Pereira OL, Kern D, Silva CA, Pereira RMR, et al. Distinct clinical correlates of immune thrombocytopenic purpura at diagnosis of childhood-onset and adult SLE. Mod Rheumatol. 2018;28:649–653. doi: 10.1080/14397595.2017.1386836.
    1. Buff Passone CG, Grisi SJ, Farhat SC, Manna T Della, Pastorino AC, Alveno RA, et al. Complexity of pediatric chronic disease: cross-sectional study with 16,237 patients followed by multiple medical specialties. Rev Paul Pediatr. 2020;38:1–8. 10.1590/1984-0462/2020/38/2018101.
    1. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SCL, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J) 2018;94:539–545. doi: 10.1016/j.jped.2017.07.014.
    1. Gormezano NWS, Silva CA, Otsuzi CI, Barros DL, Da Silva MA, Sallum AME, et al. Higher prevalence and distinct features of herpes zoster infection in children than adults with systemic lupus erythematosus. Pediatr Infect Dis J. 2015;34:905–907. doi: 10.1097/INF.0000000000000756.
    1. Gormezano NWS, Otsuzi CI, Barros DL, da Silva MA, Pereira RMR, Campos LMA, et al. Macrophage activation syndrome: A severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706–710. doi: 10.1016/j.semarthrit.2015.10.015.
    1. Gormezano NWS, Silva CA, Aikawa NE, Barros DL, da Silva MA, Otsuzi CI, et al. Chronic arthritis in systemic lupus erythematosus: distinct features in 336 paediatric and 1830 adult patients. Clin Rheumatol. 2016;35:227–231. doi: 10.1007/s10067-015-3127-3.
    1. Gormezano NWS, Kern D, Pereira OL, Esteves GCX, Sallum AME, Aikawa NE, et al. Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients. Lupus. 2017;26:426–430. doi: 10.1177/0961203316676379.
    1. Lo MS. Insights gained from the study of pediatric systemic lupus erythematosus. Front Immunol. 2018 doi: 10.3389/fimmu.2018.01278.
    1. Joo Y Bin, Park SY, Won S, Bae SC. Differences in clinical features and mortality between childhood-onset and adult-onset systemic lupus erythematosus: A prospective single-center study. J Rheumatol. 2016;43:1490–7. 10.3899/jrheum.151129.
    1. Rodsaward P, Chottawornsak N, Suwanchote S, Rachayon M, Deekajorndech T, Wright HL, Edwards SW, Beresford MW, Rerknimitr P, Chiewchengchol D. The clinical significance of antinuclear antibodies and specific autoantibodies in juvenile and adult systemic lupus erythematosus patients. Asian Pac J Allergy Immunol. 2019. 10.12932/AP-211218-0465. Epub ahead of print. PMID: 31012595.
    1. Ramos GF, Ribeiro VP, Mercadante MP, Ribeiro MP, Delgado AF, Farhat SCL, et al. Mortality in adolescents and young adults with chronic diseases during 16 years: a study in a Latin American tertiary hospital. J Pediatr (Rio J) 2019;95:667–673. doi: 10.1016/j.jped.2018.06.006.
    1. Medhat BM, Behiry ME, Sobhy N, Farag Y, Marzouk H, Mostafa N, et al. Late-onset systemic lupus erythematosus: characteristics and outcome in comparison to juvenile- and adult-onset patients—a multicenter retrospective cohort. Clin Rheumatol. 2020;39:435–442. doi: 10.1007/s10067-019-04776-y.
    1. Silva CA. Poor adherence to drug treatment in children and adolescents with autoimmune rheumatic diseases. Rev Paulista Pediatria. 2019;37:138–139. doi: 10.1590/1984-0462/;2019;37;2;00019.
    1. Farhat SCL, Yariwake VY, Veras MM, Braga ALF, Maluf AE, Silva CA. Inhaled ultrafine particles, epigenetics and systemic autoimmune rheumatic diseases. Autoimmun Rev. 2020;19:102640.
    1. Conde PG, Farhat LC, Braga ALF, Sallum AEM, Farhat SCL, Silva CA. Are prematurity and environmental factors determinants for developing childhood-onset systemic lupus erythematosus? Mod Rheumatol. 2018;28:156–160. doi: 10.1080/14397595.2017.1332508.
    1. Alves AGF, de Azevedo Giacomin MF, Braga ALF, Sallum AME, Pereira LAA, Farhat LC, et al. Influence of air pollution on airway inflammation and disease activity in childhood-systemic lupus erythematosus. Clin Rheumatol. 2018;37:683–690. doi: 10.1007/s10067-017-3893-1.
    1. Goulart MFG, Alves AGF, Farhat J, Braga ALF, Pereira LAA, de Faria Coimbra Lichtenfels AJ, et al. Influence of air pollution on renal activity in patients with childhood-onset systemic lupus erythematosus. Pediatr Nephrol. 2020;35:1247–55. 10.1007/s00467-020-04517-3.
    1. Hedrich CM, Smith EMD, Beresford MW. Juvenile-onset systemic lupus erythematosus (jSLE) – Pathophysiological concepts and treatment options. Best Pract Res Clin Rheumatol. 2017;31:488–504. doi: 10.1016/j.berh.2018.02.001.
    1. Tsokos GC. Mechanisms of disease: systemic lupus erythematosus. N Engl J Med. 2011;365:2110–21121. doi: 10.1056/NEJMra1100359.
    1. Jin O, Kavikondala S, Sun L, Fu R, Mok MY, Chan A, et al. Systemic lupus erythematosus patients have increased number of circulating plasmacytoid dendritic cells, but decreased myeloid dendritic cells with deficient CD83 expression. Lupus. 2008;17:654–662. doi: 10.1177/0961203308089410.
    1. Saferding V, Blüml S. Innate immunity as the trigger of systemic autoimmune diseases. J Autoimmun. 2020 doi: 10.1016/j.jaut.2019.102382.
    1. MacHida H, Ito S, Hirose T, Takeshita F, Oshiro H, Nakamura T, et al. Expression of toll-like receptor 9 in renal podocytes in childhood-onset active and inactive lupus nephritis. Nephrol Dial Transpl. 2010;25:2530–2537. doi: 10.1093/ndt/gfq058.
    1. An J, Minie M, Sasaki T, Woodward JJ, Elkon KB. Antimalarial drugs as immune modulators: new mechanisms for old drugs. Annu Rev Med. 2017;68:317–330. doi: 10.1146/annurev-med-043015-123453.
    1. Crow MK, Olferiev M, Kirou KA. Type i interferons in autoimmune disease. Annu Rev Pathol. 2019;14:369–393. doi: 10.1146/annurev-pathol-020117-043952.
    1. Hooks JJ, Moutsopoulos HM, Geis SA, Stahl NI, Decker JL, Notkins AL. Immune interferon in the circulation of patients with autoimmune disease. N Engl J Med. 1979;301:5–8. doi: 10.1056/nejm197907053010102.
    1. Crow MK, Type I. Interferon in the pathogenesis of lupus. J Immunol. 2014;192:5459–5468. doi: 10.4049/jimmunol.1002795.
    1. Postal M, Sinicato NA, Peliçari KO, Marini R, Costallat LTL, Appenzeller S. Clinical and serological manifestations associated with interferon-α levels in childhood-onset systemic lupus erythematosus. Clinics. 2012;67:157–162. doi: 10.6061/clinics/2012(02)11.
    1. Wahadat MJ, Bodewes ILA, Maria NI, van Helden-Meeuwsen CG, van Dijk-Hummelman A, Steenwijk EC, et al. Type I IFN signature in childhood-onset systemic lupus erythematosus: a conspiracy of DNA- and RNA-sensing receptors? Arthritis Res Ther. 2018 doi: 10.1186/s13075-017-1501-z.
    1. Obermoser G, Pascual V. The interferon-α signature of systemic lupus erythematosus. Lupus. 2010;19:1012–1019. doi: 10.1177/0961203310371161.
    1. Blanco P, Palucka AK, Gill M, Pascual V, Banchereau J. Induction of dendritic cell differentiation by IFN-α in systemic lupus erythematosus. Science. 2001;294:1540–1543. doi: 10.1126/science.1064890.
    1. Bennett L, Palucka AK, Arce E, Cantrell V, Borvak J, Banchereau J, et al. Interferon and granulopoiesis signatures in systemic lupus erythematosus blood. J Exp Med. 2003;197:711–723. doi: 10.1084/jem.20021553.
    1. Sakata K, Nakayamada S, Miyazaki Y, Kubo S, Ishii A, Nakano K, et al. Up-Regulation of TLR7-mediated IFN-α production by plasmacytoid dendritic cells in patients with systemic lupus erythematosus. Front Immunol. 2018;9:1957. doi: 10.3389/fimmu.2018.01957.
    1. Rönnblom L, Alm GV. Systemic lupus erythematosus and the type I interferon system. Arthritis Res Ther. 2003;5:68–75. doi: 10.1186/ar625.
    1. Liphaus BL, Lima L, Palmeira P, Silva CA, Goldenstein-Schainberg C, Carneiro-Sampaio M. Increased sMer, but not sAxl, sTyro3, and Gas6 relate with active disease in juvenile systemic lupus erythematosus. Clin Rheumatol. 2020;39:509–514. doi: 10.1007/s10067-019-04799-5.
    1. Jesus AA, Liphaus BL, Silva CA, Bando SY, Andrade L, Coutinho A, et al. Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients. Lupus. 2011;20:1275–1284. doi: 10.1177/0961203311411598.
    1. Macedo ACL, Isaac L. Systemic lupus erythematosus and deficiencies of early components of the complement classical pathway. Front Immunol. 2016 doi: 10.3389/fimmu.2016.00055.
    1. Liphaus BL, Umetsu N, Jesus AA, Bando SY, Silva CA, Carneiro-Sampaio M. Molecular characterization of the complement C1q, C2 and C4 genes in Brazilian patients with juvenile systemic lupus erythematosus. Clinics. 2015;70:220–227. doi: 10.6061/clinics/2015(03)12.
    1. Jesus AJ, Silva CA, Carneiro-Sampaio M, Sheinberg M, Mangueira CL, Marie SK, et al. Anti-C1q antibodies in juvenile-onset systemic lupus erythematosus. In: Annals of the New York Academy of Sciences. Blackwell Publishing Inc.; 2009. pp. 235–8. 10.1111/j.1749-6632.2009.04675.x.
    1. Mak A, Kow NY. The pathology of t cells in systemic lupus erythematosus. J Immunol Res. 2014 doi: 10.1155/2014/419029.
    1. Tsokos GC, Lo MS, Reis PC, Sullivan KE. New insights into the immunopathogenesis of systemic lupus erythematosus. Nat Rev Rheumatol. 2016;12:716–730. doi: 10.1038/nrrheum.2016.186.
    1. Singla S, Wenderfer SE, Muscal E, Sagcal-Gironella ACP, Orange JS, Makedonas G. Changes in frequency and activation status of major CD4+ T-cell subsets after initiation of immunosuppressive therapy in a patient with new diagnosis childhood-onset systemic lupus erythematosus. Front Pediatr. 2017 doi: 10.3389/fped.2017.00104.
    1. Smith EMD, Lythgoe H, Midgley A, Beresford MW, Hedrich CM. Juvenile-onset systemic lupus erythematosus: update on clinical presentation, pathophysiology and treatment options. Clin Immunol. 2019 doi: 10.1016/j.clim.2019.108274.
    1. Enyedy EJ, Nambiar MP, Liossis SNC, Dennis G, Kammer GM, Tsokos GC. Fcε receptor type I γ chain replaces the deficient T cell receptor ζ chain in T cells of patients with systemic lupus erythematosus. Arthritis Rheum. 2001;44:1114–1121. doi: 10.1002/1529-0131(200105)44:5<1114::AID-ANR192>;2-B.
    1. Hong SD, Reiff A, Yang HT, Migone TS, Ward CD, Marzan K, et al. B lymphocyte stimulator expression in pediatric systemic lupus erythematosus and juvenile idiopathic arthritis patients. Arthritis Rheum. 2009;60:3400–3409. doi: 10.1002/art.24902.
    1. Deafen D, Escalante A, Weinrib L, Horwitz D, Bachman B, Roy-Burman P, et al. A revised estimate of twin concordance in systemic lupus erythematosus. Arthritis Rheum. 1992;35:311–318. doi: 10.1002/art.1780350310.
    1. Dominguez D, Kamphuis S, Beyene J, Wither J, Harley JB, Blanco I, et al. Relationship between genetic risk and age of diagnosis of systemic lupus erythematosus. J Rheumatol. 2020 doi: 10.3899/jrheum.200002.
    1. Chen L, Morris DL, Vyse TJ. Genetic advances in systemic lupus erythematosus. Curr Opin Rheumatol. 2017;29:423–433. doi: 10.1097/BOR.0000000000000411.
    1. Webber D, Cao J, Dominguez D, Gladman DD, Levy DM, Ng L, et al. Association of systemic lupus erythematosus (SLE) genetic susceptibility loci with lupus nephritis in childhood-onset and adult-onset SLE. Rheumatology. 2020;59:90–98. doi: 10.1093/rheumatology/kez220.
    1. Liphaus BL, Caramalho I, Rangel-Santos A, Silva CA, Demengeot J, Carneiro-Sampaio MMS. LRBA deficiency: A new genetic cause of monogenic lupus. Ann Rheum Dis. 2019 doi: 10.1136/annrheumdis-2019-216410.
    1. Demirkaya E, Sahin S, Romano M, Zhou Q, Aksentijevich I. New horizons in the genetic etiology of systemic lupus erythematosus and lupus-like disease: monogenic lupus and beyond. J Clin Med. 2020;9:712. doi: 10.3390/jcm9030712.
    1. Costa-Reis P, Sullivan KE. Monogenic lupus: it’s all new! Curr Opin Immunol. 2017;49:87–95. doi: 10.1016/j.coi.2017.10.008.
    1. Bousfiha A, Jeddane L, Picard C, Al-Herz W, Ailal F, Chatila T, et al. Human inborn errors of immunity: 2019 update of the IUIS Phenotypical Classification. J Clin Immunol. 2020;40:66–81. doi: 10.1007/s10875-020-00758-x.
    1. Alperin JM, Ortiz-Fernández L, Sawalha AH. Monogenic lupus: a developing paradigm of disease. Front Immunol. 2018;9:2496. doi: 10.3389/fimmu.2018.02496.
    1. Carneiro-Sampaio M, Coutinho A. Early-onset autoimmune disease as a manifestation of primary immunodeficiency. Front Immunol. 2015 doi: 10.3389/fimmu.2015.00185.
    1. Rivas-Larrauri F, Yamazaki-Nakashimada MA. Lupus eritematoso sistémico: ¿es una sola enfermedad? Reumatol Clin. 2016;12:274–281. doi: 10.1016/j.reuma.2016.01.005.
    1. Figgett WA, Monaghan K, Ng M, Alhamdoosh M, Maraskovsky E, Wilson NJ, et al. Machine learning applied to whole-blood RNA-sequencing data uncovers distinct subsets of patients with systemic lupus erythematosus. Clin Transl Immunol. 2019 doi: 10.1002/cti2.1093.
    1. Massias JS, Smith EMD, Al-Abadi E, Armon K, Bailey K, Ciurtin C, et al. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups. Lupus. 2020;29:474–481. doi: 10.1177/0961203320909156.
    1. Rubinstein TB, Mowrey WB, Ilowite NT, Wahezi DM, Abramson L, Anderson E, et al. Delays to care in pediatric lupus patients: data from the childhood arthritis and rheumatology research alliance legacy registry. Arthritis Care Res. 2018;70:420–427. doi: 10.1002/acr.23285.
    1. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725. doi: 10.1002/art.1780400928.
    1. Petri M, Orbai AM, Alarcõn GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677–2686. doi: 10.1002/art.34473.
    1. Lythgoe H, Morgan T, Heaf E, Lloyd O, Al-Abadi E, Armon K, et al. Evaluation of the ACR and SLICC classification criteria in juvenile-onset systemic lupus erythematosus: a longitudinal analysis. Lupus. 2017;26:1285–1290. doi: 10.1177/0961203317700484.
    1. Tao JJ, Hiraki LT, Levy DM, Silverman ED. Comparison of sensitivities of American college of rheumatology and systemic lupus international collaborating clinics classification criteria in childhood-onset systemic lupus erythematosus. J Rheumatol. 2019;46:731–738. doi: 10.3899/jrheum.180337.
    1. Sag E, Tartaglione A, Batu ED, Ravelli A, Khalil SMA, Marks SD, et al. Performance of the new SLICC classification criteria in childhood systemic lupus erythematosus: a multicentre study. Clin Exp Rheumatol. 2014;32:440–444.
    1. Fonseca AR, Gaspar-Elsas MIC, Land MGP, De Oliveira SKF. Comparison between three systems of classification criteria in juvenile systemic lupus erythematous. Rheumatology. 2015;54:241–247. doi: 10.1093/rheumatology/keu278.
    1. Hartman EAR, van Royen-Kerkhof A, Jacobs JWG, Welsing PMJ, Fritsch-Stork RDE. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-analysis. Autoimmun Rev. 2018;17:316–322. doi: 10.1016/j.autrev.2018.01.007.
    1. Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis. 2019;78:1151–1159. doi: 10.1136/annrheumdis-2018-214819.
    1. Aljaberi N, Nguyen K, Strahle C, Merritt A, Mathur A, Brunner HI. The Performance of the New 2019-EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus in Children and Young Adults. Arthritis Care Res. 2020 doi: 10.1002/acr.24430.
    1. Ma M, Hui-Yuen JS, Cerise JE, Iqbal S, Eberhard BA. Validation of the 2019 European League Against Rheumatism/American College of Rheumatology Criteria Compared to the 1997 American College of Rheumatology Criteria and the 2012 Systemic Lupus International Collaborating Clinics Criteria in Pediatric Systemic Lupus Erythematosus. Arthritis Care Res. 2020;72:1597–1601. doi: 10.1002/acr.24057.
    1. Fonseca AR, Rodrigues MCF, Sztajnbok FR, Land MGP, De Oliveira SKF. Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus. Adv Rheumatol. 2019 doi: 10.1186/s42358-019-0062-z.
    1. Lattanzi B, Consolaro A, Solari N, Ruperto N, Martini A, Ravelli A. Measures of disease activity and damage in pediatric systemic lupus erythematosus: British Isles Lupus Assessment Group (BILAG), European Consensus Lupus Activity Measurement (ECLAM), Systemic Lupus Activity Measure (SLAM), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Physician’s Global Assessment of Disease Activity (MD Global), and Systemic Lupus International Collaborating. Arthritis Care Res. 2011;63(SUPPL):11. doi: 10.1002/acr.20623.
    1. Guariento A, Silva MFC, Tassetano PSF, Rocha SMS, Campos LMA, Valente M, et al. Biometria do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico. Rev Bras Reumatol. 2015;55:346–351. doi: 10.1016/j.rbr.2014.12.010.
    1. Groot N, De Graeff N, Avcin T, Bader-Meunier B, Brogan P, Dolezalova P, et al. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: The SHARE initiative. Ann Rheum Dis. 2017;76:1788–1796. doi: 10.1136/annrheumdis-2016-210960.
    1. Groot N, De Graeff N, Marks SD, Brogan P, Avcin T, Bader-Meunier B, et al. European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: The SHARE initiative. Ann Rheum Dis. 2017;76:1965–1973. doi: 10.1136/annrheumdis-2017-211898.
    1. Franklyn K, Lau CS, Navarra SV, Louthrenoo W, Lateef A, Hamijoyo L, et al. Definition and initial validation of a Lupus Low Disease Activity State (LLDAS) Ann Rheum Dis. 2016;75:1615–1621. doi: 10.1136/annrheumdis-2015-207726.
    1. Golder V, Tsang-A-Sjoe MWP. Treatment targets in SLE: Remission and low disease activity state. Rheumatology. 2020;59:V19–28. doi: 10.1093/rheumatology/keaa420.
    1. Golder V, Kandane-Rathnayake R, Huq M, Nim HT, Louthrenoo W, Luo SF, et al. Lupus low disease activity state as a treatment endpoint for systemic lupus erythematosus: a prospective validation study. Lancet Rheumatol. 2019;1:e95–102. doi: 10.1016/S2665-9913(19)30037-2.
    1. Brunner HI, Holland M, Beresford MW, Ardoin SP, Appenzeller S, Silva CA, et al. American College of Rheumatology Provisional Criteria for global flares in childhood-onset systemic lupus erythematosus. Arthritis Care Res. 2018;70:813–822. doi: 10.1002/acr.23557.
    1. Brunner HI, Holland MJ, Beresford MW, Ardoin SP, Appenzeller S, Silva CA, et al. American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus. Arthritis Care Res. 2019;71:579–590. doi: 10.1002/acr.23834.
    1. Gutiérrez-Suárez R, Ruperto N, Gastaldi R, Pistorio A, Felici E, Burgos-Vargas R, et al. A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus. Arthritis Rheum. 2006;54:2989–2996. doi: 10.1002/art.22048.
    1. Watson L, Leone V, Pilkington C, Tullus K, Rangaraj S, McDonagh JE, et al. Disease activity, severity, and damage in the UK Juvenile-onset systemic lupus erythematosus cohort. Arthritis Rheum. 2012;64:2356–2365. doi: 10.1002/art.34410.
    1. Lim LSH, Lefebvre A, Benseler S, Silverman ED. Longterm outcomes and damage accrual in patients with childhood systemic lupus erythematosus with psychosis and severe cognitive dysfunction. J Rheumatol. 2013;40:513–519. doi: 10.3899/jrheum.121096.
    1. Holland MJ, Beresford MW, Feldman BM, Huggins J, Norambuena X, Silva CA, et al. Measuring disease damage and its severity in childhood-onset systemic lupus erythematosus. Arthritis Care Res. 2018;70:1621–1629. doi: 10.1002/acr.23531.
    1. Salah S, Lotfy HM, Mokbel AN, Kaddah AM, Fahmy N. Damage index in childhood-onset systemic lupus erythematosus in Egypt. Pediatr Rheumatol. 2011 doi: 10.1186/1546-0096-9-36.
    1. Lim LSH, Pullenayegum E, Lim L, Gladman D, Feldman B, Silverman E. From childhood to adulthood: the trajectory of damage in patients with juvenile-onset systemic lupus erythematosus. Arthritis Care Res. 2017;69:1627–1635. doi: 10.1002/acr.23199.
    1. Groot N, Shaikhani D, Teng YKO, de Leeuw K, Bijl M, Dolhain RJEM, et al. Long-term clinical outcomes in a cohort of adults with childhood-onset systemic lupus erythematosus. Arthritis Rheumatol. 2019;71:290–301. doi: 10.1002/art.40697.
    1. Pons-Estel GJ, Ugarte-Gil MF, Alarcón GS. Epidemiology of systemic lupus erythematosus. Expert Rev Clin Immunol. 2017;13:799–814. doi: 10.1080/1744666X.2017.1327352.
    1. Brunner HI, Martini A, Lovell DJ, Ruperto N. Clinical trials in children and adolescents with systemic lupus erythematosus: Methodological aspects, regulatory landscape and future opportunities. Ann Rheum Dis. 2019;78:162–170. doi: 10.1136/annrheumdis-2018-213198.
    1. Rubin HR, Pronovost P, Diette GB. The advantages and disadvantages of process-based measures of health care quality. Int J Qual Heal Care. 2001;13:469–474. doi: 10.1093/intqhc/13.6.469.
    1. Hollander MC, Sage JM, Greenler AJ, Pendl J, Avcin T, Espada G, et al. International consensus for provisions of quality-driven care in childhood-onset systemic lupus erythematosus. Arthritis Care Res. 2013;65:1416–1423. doi: 10.1002/acr.21998.
    1. Mina R, Harris JG, Klein-Gitelman MS, Appenzeller S, Centeville M, Eskra D, et al. Initial benchmarking of the quality of medical care in childhood-onset systemic lupus erythematosus. Arthritis Care Res. 2016;68:179–186. doi: 10.1002/acr.22666.
    1. Silva CA, Aikawa NE, Bonfa E. Vaccinations in juvenile chronic inflammatory diseases: an update. Nat Rev Rheumatol. 2013;9:532–543. doi: 10.1038/nrrheum.2013.95.
    1. Rotstein Grein IH, Pinto NF, Lobo A, Groot N, Sztajnbok F, da Silva CAA, et al. Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with childhood systemic lupus erythematosus: a real-world interventional multi-centre study. Lupus. 2020;29:934–942. doi: 10.1177/0961203320928406.
    1. Groot N, Heijstek MW, Wulffraat NM. Vaccinations in paediatric rheumatology: an update on current developments. Curr Rheumatol Rep. 2015;17:46.
    1. Schulze-Koops H, Specker C, Skapenko A. Vaccination of patients with inflammatory rheumatic diseases against SARS-CoV-2: considerations before widespread availability of the vaccines. RMD Open. 2021;7(1):e001553. 10.1136/rmdopen-2020-001553
    1. Tang W, Askanase AD, Khalili L, Merrill JT. SARS-CoV-2 vaccines in patients with SLE. Lupus Sci Med. 2021;8(1):e000479.10.1136/lupus-2021-000479
    1. Wallace M, Woodworth KR, Gargano JW, Scobie HM, Blain AE, Moulia D, et al. The Advisory Committee on Immunization Practices’ Interim Recommendation for Use of Pfizer-BioNTech COVID-19 Vaccine in Adolescents Aged 12–15 Years—United States, May 2021. MMWR Morb Mortal Wkly Rep. 2021;70:749–752. doi: 10.15585/mmwr.mm7020e1.
    1. Kelvin AA, Halperin S. COVID-19 in children: the link in the transmission chain. Lancet Infect Dis. 2020;20:633–634. doi: 10.1016/S1473-3099(20)30236-X.
    1. Wang E, Brar K. COVID-19 in Children: an epidemiology study from China. J Allergy Clin Immunol Pract. 2020;8:2118–2120. doi: 10.1016/j.jaip.2020.04.024.
    1. Avar Aydin PO, Shan J, Brunner HI, Mitsnefes MM. Blood pressure control over time in childhood-onset systemic lupus erythematous. Lupus. 2018;27:657–664. doi: 10.1177/0961203317751061.
    1. Fanouriakis A, Kostopoulou M, Cheema K, Anders HJ, Aringer M, Bajema I, et al. 2019 Update of the Joint European League against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis. Ann Rheum Dis. 2020;79:S713–S723. doi: 10.1136/annrheumdis-2020-216924.
    1. Ugolini-Lopes MR, Seguro LPC, Castro MXF, Daffre D, Lopes AC, Borba EF, et al. Early proteinuria response: a valid real-life situation predictor of long-term lupus renal outcome in an ethnically diverse group with severe biopsy-proven nephritis? Lupus Sci Med. 2017 doi: 10.1136/lupus-2017-000213.
    1. Castro M, Ugolini-Lopes M, Borba EF, Bonfá E, Seguro LPC. Effectiveness of renoprotective approaches for persistent proteinuria in lupus nephritis: more than just immunosuppression. Lupus. 2018;27:2215–2219. doi: 10.1177/0961203318809883.
    1. Klumb EM, Silva CAA, Lanna CCD, Sato EI, Borba EF, Brenol JCT, et al. Consenso da Sociedade Brasileira de Reumatologia para o diagnóstico, manejo e tratamento da nefrite lúpica. Rev Bras Reumatol. 2015;55:1–21. doi: 10.1016/j.rbr.2014.09.008.
    1. Gordon C, Amissah-Arthur MB, Gayed M, Brown S, Bruce IN, D’Cruz D, et al. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults. Rheumatology (Oxford) 2018;57:e1–45. doi: 10.1093/rheumatology/kex286.
    1. Gualano B, Bonfa E, Pereira RMR, Silva CA. Physical activity for paediatric rheumatic diseases: standing up against old paradigms. Nat Rev Rheumatol. 2017;13:368–379. doi: 10.1038/nrrheum.2017.75.
    1. Pinto AJ, Miyake CNH, Benatti FB, Silva CA, Sallum AME, Borba E, et al. Reduced aerobic capacity and quality of life in physically inactive patients with systemic lupus erythematosus with mild or inactive disease. Arthritis Care Res. 2016;68:1780–1786. doi: 10.1002/acr.22905.
    1. Putera AM, Irwanto I, Maramis MM, Prasetyo RV, Soemyarso NA, Noer MS. Effect of mental health problems on the quality of life in children with lupus nephritis. Neuropsychiatr Dis Treat. 2020;16:1583–1593. doi: 10.2147/NDT.S250373.
    1. Knight AM, Vickery ME, Muscal E, Davis AM, Harris JG, Soybilgic A, et al. Identifying targets for improving mental healthcare of adolescents with systemic lupus erythematosus: perspectives from pediatric rheumatology clinicians in the United States and Canada. J Rheumatol. 2016;43:1136–1145. doi: 10.3899/jrheum.151228.
    1. Jones JT, Cunningham N, Kashikar-Zuck S, Brunner HI. Pain, fatigue, and psychological impact on health-related quality of life in childhood-onset lupus. Arthritis Care Res. 2016;68:73–80. doi: 10.1002/acr.22650.
    1. Rodriguez-Smith J, Brunner HI. Update on the treatment and outcome of systemic lupus erythematous in children. Curr Opin Rheumatol. 2019;31:464–470. doi: 10.1097/BOR.0000000000000621.
    1. Ponticelli C, Moroni G. Hydroxychloroquine in systemic lupus erythematosus (SLE) Expert Opin Drug Saf. 2017;16:411–419. doi: 10.1080/14740338.2017.1269168.
    1. Harris JG, Maletta KI, Kuhn EM, Olson JC. Evaluation of quality indicators and disease damage in childhood-onset systemic lupus erythematosus patients. Clin Rheumatol. 2017;36:351–359. doi: 10.1007/s10067-016-3518-0.
    1. Marmor MF, Kellner U, Lai TYY, Melles RB, Mieler WF, Lum F. Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy (2016 Revision) Ophthalmology. 2016;123:1386–1394. doi: 10.1016/j.ophtha.2016.01.058.
    1. Deng J, Chalhoub NE, Sherwin CM, Li C, Brunner HI. Glucocorticoids pharmacology and their application in the treatment of childhood-onset systemic lupus erythematosus. Semin Arthritis Rheum. 2019;49:251–259. doi: 10.1016/j.semarthrit.2019.03.010.
    1. Guiducci C, Gong M, Xu Z, Gill M, Chaussabel D, Meeker T, et al. TLR recognition of self nucleic acids hampers glucocorticoid activity in lupus. Nature. 2010;465:937–941. doi: 10.1038/nature09102.
    1. Heshin-Bekenstein M, Trupin L, Yelin E, von Scheven E, Yazdany J, Lawson EF. Longitudinal disease- and steroid-related damage among adults with childhood-onset systemic lupus erythematosus. Semin Arthritis Rheum. 2019;49:267–272. doi: 10.1016/j.semarthrit.2019.05.010.
    1. Sawah S Al, Zhang X, Zhu B, Magder LS, Foster SA, Iikuni N, et al. Effect of corticosteroid use by dose on the risk of developing organ damage over time in systemic lupus erythematosus-the Hopkins Lupus Cohort. Lupus Sci Med. 2015;2:66. 10.1136/lupus-2014-000066.
    1. Dye AM, Nelson GB, Diaz-Thomas A. Delayed puberty. Pediatr Ann. 2018;47:e16–22. doi: 10.3928/19382359-20171215-01.
    1. Jongvilaikasem P, Rianthavorn P. Longitudinal growth patterns and final height in childhood-onset systemic lupus erythematosus. Eur J Pediatr. 2021;180:1431–1441. doi: 10.1007/s00431-020-03910-2.
    1. Rygg M, Pistorio A, Ravelli A, Maghnie M, Di IN, Bader-Meunier B, et al. A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus. Ann Rheum Dis. 2012;71:511–517. doi: 10.1136/annrheumdis-2011-200106.
    1. Peterknecht E, Keasey MP, Beresford MW. The effectiveness and safety of biological therapeutics in juvenile-onset systemic lupus erythematosus (JSLE): a systematic review. Lupus. 2018;27:2135–2145. doi: 10.1177/0961203318804879.
    1. Neely J, von Scheven E. Autoimmune haemolytic anaemia and autoimmune thrombocytopenia in childhood-onset systemic lupus erythematosus: updates on pathogenesis and treatment. Curr Opin Rheumatol. 2018;30:498–505. doi: 10.1097/BOR.0000000000000523.
    1. Correll CK, Miller DD, Maguiness SM. Treatment of Childhood-Onset Lupus Erythematosus Panniculitis with Rituximab. JAMA Dermatol. 2020;156:566–569. doi: 10.1001/jamadermatol.2019.4984.
    1. AlE’ed A, AlSonbul A, Al-Mayouf SM. Safety and efficacy of combined cyclophosphamide and rituximab treatment in recalcitrant childhood lupus. Rheumatol Int. 2014;34:529–33.
    1. Speth F, Hinze CH, Schranz P, Miller-Wiegart E, Haefner R. Combination of rituximab and abatacept as an exit strategy for repetitive B-cell depletion in children with severe autoimmune diseases: a report of three cases. Lupus. 2018;27:1996–1998. doi: 10.1177/0961203318783057.
    1. Yu T, Enioutina EY, Brunner HI, Vinks AA, Sherwin CM. Clinical pharmacokinetics and pharmacodynamics of biologic therapeutics for treatment of systemic lupus erythematosus. Clin Pharmacokinet. 2017;56:107–125. doi: 10.1007/s40262-016-0426-z.
    1. Brunner HI, Abud-Mendoza C, Viola DO, Calvo Penades I, Levy D, Anton J, et al. Safety and efficacy of intravenous belimumab in children with systemic lupus erythematosus: results from a randomised, placebo-controlled trial. Ann Rheum Dis. 2020;79:1340–1348. doi: 10.1136/annrheumdis-2020-217101.
    1. Shah K, Cragg M, Leandro M, Reddy V. Anti-CD20 monoclonal antibodies in systemic lupus erythematosus. Biologicals. 2021;69:1–14. doi: 10.1016/j.biologicals.2020.11.002.
    1. Khamashta M, Merrill JT, Werth VP, Furie R, Kalunian K, Illei GG, et al. Sifalimumab, an anti-interferon-α monoclonal antibody, in moderate to severe systemic lupus erythematosus: a randomised, double-blind, placebo-controlled study. Ann Rheum Dis. 2016;75:1909–1916. doi: 10.1136/annrheumdis-2015-208562.
    1. Furie R, Khamashta M, Merrill JT, Werth VP, Kalunian K, Brohawn P, et al. Anifrolumab, an anti–interferon-α receptor monoclonal antibody, in moderate-to-severe systemic lupus erythematosus. Arthritis Rheumatol. 2017;69:376–386. doi: 10.1002/art.39962.
    1. Tocut M, Shoenfeld Y, Zandman-Goddard G. Systemic lupus erythematosus: an expert insight into emerging therapy agents in preclinical and early clinical development. Expert Opin Investig Drugs. 2020;29:1151–1162. doi: 10.1080/13543784.2020.1807004.
    1. Cinar OK, Marlais M, Al Obaidi M, Cheng IL, Tullus K, Brogan P, et al. Ofatumumab use in juvenile systemic lupus erythematosus: a single centre experience. Lupus. 2021;30:527–530. doi: 10.1177/0961203320981137.
    1. Pin A, Tesser A, Pastore S, Moressa V, Valencic E, Arbo A, et al. Biological and clinical changes in a pediatric series treated with off-label JAK inhibitors. Int J Mol Sci. 2020;21:1–29. doi: 10.3390/ijms21207767.
    1. Mok CC. Calcineurin inhibitors in systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2017;31:429–438. doi: 10.1016/j.berh.2017.09.010.
    1. Liu Z, Zhang H, Liu Z, Xing C, Fu P, Ni Z, et al. Multitarget therapy for induction treatment of lupus nephritis: a randomized trial. Ann Intern Med. 2015;162:18–26. doi: 10.7326/M14-1030.
    1. Rovin BH, Solomons N, Pendergraft WF, Dooley MA, Tumlin J, Romero-Diaz J, et al. A randomized, controlled double-blind study comparing the efficacy and safety of dose-ranging voclosporin with placebo in achieving remission in patients with active lupus nephritis. Kidney Int. 2019;95:219–231. doi: 10.1016/j.kint.2018.08.025.
    1. Palmer SC, Tunnicliffe DJ, Singh-Grewal D, Mavridis D, Tonelli M, Johnson DW, et al. Induction and maintenance immunosuppression treatment of proliferative lupus nephritis: a network meta-analysis of randomized trials. Am J Kidney Dis. 2017;70:324–336. doi: 10.1053/j.ajkd.2016.12.008.
    1. Mok CC, Ying KY, Yim CW, Siu YP, Tong KH, To CH, et al. Tacrolimus versus mycophenolate mofetil for induction therapy of lupus nephritis: a randomised controlled trial and long-term follow-up. Ann Rheum Dis. 2016;75:30–36. doi: 10.1136/annrheumdis-2014-206456.
    1. Tiseo BC, Cocuzza M, Bonfá E, Srougi M, Silva CA. Male fertility potential alteration in rheumatic diseases: a systematic review. Int Braz J Urol. 2016;42:11–21. doi: 10.1590/S1677-5538.IBJU.2014.0595.
    1. Parodis I, Tamirou F, Houssiau FA. Prediction of prognosis and renal outcome in lupus nephritis. Lupus Sci Med. 2020 doi: 10.1136/lupus-2020-000389.
    1. Wenderfer SE, Ruth NM, Brunner HI. Advances in the care of children with lupus nephritis. Pediatr Res. 2017;81:406–414. doi: 10.1038/pr.2016.247.
    1. Dall’Era M, Cisternas MG, Smilek DE, Straub L, Houssiau FA, Cervera R, et al. Predictors of long-term renal outcome in lupus nephritis trials: lessons learned from the euro-lupus nephritis cohort. Arthritis Rheumatol. 2015;67:1305–13. 10.1002/art.39026.
    1. Tamirou F, Lauwerys BR, Dall’Era M, Mackay M, Rovin B, Cervera R, et al. A proteinuria cut-off level of 0.7 g /day after 12 months of treatment best predicts long-term renal outcome in lupus nephritis: Data from the MAINTAIN Nephritis Trial. Lupus Sci Med. 2015. 10.1136/lupus-2015-000123.
    1. Mackay M, Dall’Era M, Fishbein J, Kalunian K, Lesser M, Sanchez-Guerrero J, et al. Establishing surrogate kidney end points for lupus nephritis clinical trials: development and validation of a novel approach to predict future kidney outcomes. Arthritis Rheumatol. 2019;71:411–9. 10.1002/art.40724.
    1. Baqi N, Moazami S, Singh A, Ahmad H, Balachandra S, Tejani A. Lupus nephritis in children: a longitudinal study of prognostic factors and therapy. J Am Soc Nephrol. 1996;7:924–9.
    1. Mahajan A, Amelio J, Gairy K, Kaur G, Levy RA, Roth D, et al. Systemic lupus erythematosus, lupus nephritis and end-stage renal disease: a pragmatic review mapping disease severity and progression. Lupus. 2020;29:1011–1020. doi: 10.1177/0961203320932219.
    1. Kavanaugh AF, Solomon DH, Schur P, Reveille JD, Sherrer YRS, Lahita R. Guidelines for immunologic laboratory testing in the rheumatic diseases: anti-DNA antibody tests. Arthritis Care Res. 2002;47:546–555. doi: 10.1002/art.10558.
    1. Watson L, Tullus K, Pilkington C, Chesters C, Marks SD, Newland P, et al. Urine biomarkers for monitoring juvenile lupus nephritis: a prospective longitudinal study. Pediatr Nephrol. 2014;29:397–405. doi: 10.1007/s00467-013-2668-4.
    1. Brunner HI, Gulati G, Klein-Gitelman MS, Rouster-Stevens KA, Tucker L, Ardoin SP, et al. Urine biomarkers of chronic kidney damage and renal functional decline in childhood-onset systemic lupus erythematosus. Pediatr Nephrol. 2019;34:117–128. doi: 10.1007/s00467-018-4049-5.
    1. Harry O, Crosby LE, Smith AW, Favier L, Aljaberi N, Ting TV, et al. Self-management and adherence in childhood-onset systemic lupus erythematosus: what are we missing? Lupus. 2019;28:642–650. doi: 10.1177/0961203319839478.
    1. van Weelden M, Queiroz LB, Lourenço DMR, Kozua K, Lourenço B, Silva CA. Alcohol, smoking and illicit drug use in pediatric systemic lupus erythematosus patients. Rev Bras Reumatol. 2016;56:228–234. doi: 10.1016/j.rbre.2016.02.001.
    1. Tunnicliffe DJ, Singh-Grewal D, Chaitow J, MacKie F, Manolios N, Lin MW, et al. Lupus means sacrifices: perspectives of adolescents and young adults with systemic lupus erythematosus. Arthritis Care Res. 2016;68:828–837. doi: 10.1002/acr.22749.
    1. Davis AM, Graham TB, Zhu Y, McPheeters ML. Depression and medication nonadherence in childhood-onset systemic lupus erythematosus. Lupus. 2018;27:1532–1541. doi: 10.1177/0961203318779710.
    1. Bugni VM, Ozaki LS, Okamoto KYK, Barbosa CMPL, Hilário MOE, Len CA, et al. Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases. J Pediatr (Rio J) 2012;88:483–488. doi: 10.2223/JPED.2227.
    1. Oliveira-Santos M, Verani JFS, Klumb EM, Albuquerque EMN. Evaluation of adherence to drug treatment in patients with systemic lupus erythematosus in Brazil. Lupus. 2011;20:320–329. doi: 10.1177/0961203310383736.
    1. Feldman CH, Yazdany J, Guan H, Solomon DH, Costenbader KH. Medication nonadherence is associated with increased subsequent acute care utilization among medicaid beneficiaries with systemic lupus erythematosus. Arthritis Care Res. 2015;67:1712–1721. doi: 10.1002/acr.22636.
    1. Miotto e Silva VB, Okamoto KYK, Da Silva Ozaki L, Len CA, Sande e Lemos Ramos Ascensão Terreri MT de. Early detection of poor adherence to treatment of pediatric rheumatic diseases: pediatric rheumatology adherence questionnaire—a pilot study. Rev Paul Pediatr. 2019;37:149–55. 10.1590/1984-0462/;2019;37;2;00015.
    1. Scalzi LV, Hollenbeak CS, Mascuilli E, Olsen N. Improvement of medication adherence in adolescents and young adults with SLE using web-based education with and without a social media intervention, a pilot study. Pediatr Rheumatol. 2018;16:18. doi: 10.1186/s12969-018-0232-2.
    1. Dantas LO, Weber S, Osani MC, Bannuru RR, McAlindon TE, Kasturi S. Mobile health technologies for the management of systemic lupus erythematosus: a systematic review. Lupus. 2020;29:144–156. doi: 10.1177/0961203319897139.

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